ABSTRACT
Familial eruptive syringoma (FES) is an uncommon clinical presentation of syringoma, a benign tumour of the intraepidermal portion of the eccrine sweat ducts. It is characterized by firm, smooth, skin-coloured to pigmented, discrete papules that appear as successive crops on the anterior body surface of individuals who also have one or more family member(s) with similar eruptive or localized lesions. The inheritance is autosomal dominant. Eight types of familial syringomas have been proposed, although the number of reported cases is quite few. We present a case of familial eruptive syringoma that could be classified as type 4 familial syringoma.
Subject(s)
Syringoma/classification , Syringoma/genetics , Adolescent , Biopsy , Female , Genes, Dominant/genetics , Humans , Sclerosis/etiology , Skin/pathologyABSTRACT
BACKGROUND: Syringomas are benign neoplasms of eccrine origin. A clinical variant is eruptive syringomas, which presents as firm, smooth, yellow to pigmented papules that appear as successive crops on the neck, axillae, chest, abdomen, and/or periumbilical region. To our knowledge, there are only 10 published reports of familial eruptive syringomas. Herein we describe the eleventh report of familial eruptive syringomas, review the literature on this unusual presentation, and suggest a novel classification of familial syringomas based on our literature review. OBSERVATIONS: We report two cases of eruptive syringoma within a family. Eruptive syringomas were widely distributed on the trunk of a healthy 16-year-old female and her 19-year-old brother. Both the 19-year-old man and his mother also had infraorbital syringomas. CONCLUSION: Familial eruptive syringomas are a rare clinical entity that is likely autosomal dominantly inherited. Future reports of this unusual condition may provide further insight into the etiology of familial syringomas, and genetic analysis of cases may enable the causative gene mutation to be determined.
Subject(s)
Sweat Gland Neoplasms/genetics , Sweat Gland Neoplasms/pathology , Syringoma/genetics , Syringoma/pathology , Adolescent , Adult , Female , Humans , Male , Sweat Gland Neoplasms/classification , Syringoma/classification , Young AdultSubject(s)
Breast Neoplasms/diagnosis , Carcinoma, Adenosquamous/diagnosis , Aged , Breast Diseases/diagnosis , Breast Diseases/pathology , Breast Neoplasms/pathology , Carcinoma, Adenosquamous/pathology , Diagnosis, Differential , Female , Fibrosis , Humans , Metaplasia , Prognosis , Sclerosis , Stromal Cells/pathology , Syringoma/classification , Syringoma/pathologyABSTRACT
No disponible
Subject(s)
Adult , Female , Humans , Histocytological Preparation Techniques , Biopsy/methods , Metaplasia/diagnosis , Metaplasia/surgery , Metaplasia/etiology , Adenoma, Pleomorphic/diagnosis , Adenoma, Pleomorphic/etiology , Adenoma, Pleomorphic/pathology , Syringoma/classification , Syringoma/diagnosis , Syringoma/pathology , Adenoma/diagnosis , Adenoma/etiology , Clinical Diagnosis , Skin Neoplasms/surgery , Skin Neoplasms/complications , Skin Neoplasms/diagnosis , Skin Neoplasms/etiologyABSTRACT
We describe a unique presentation of multiple unilateral syringomas characterized by indurated plaques. Histologically, in addition to typical eccrine duct proliferation, desmoplasia exceeding by far that usually observed in syringoma, accounted for the hard consistency of the plaques. This unusual presentation should be added to the numerous clinical variants of syringoma.
